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UK CF Registry at-a-glance report 2018

Active patients

There are 10,509 active patients currently recorded on the Registry. Active patients are people who are currently alive and have had an annual review recorded in the last three years. 9,847 annual reviews were recorded in 2018.

Median age

20 is the median age of people with CF in the UK. 60.4% of the population are aged 16 or over, and 56.2% of the population are aged 18 or over.

Mode of presentation

2,751 (27.9%) people were diagnosed by newborn screening. Aside from newborn screening, the most common three presentations were:

Diagnosis

23 days is the median age that people aged under 16 in 2018 were diagnosed with cystic fibrosis. 8.5% of people in the Registry were diagnosed at age 16 or over.

CF population by devolved nation

This map shows how many people with cystic fibrosis there are living in England, Scotland, Wales and Northern Ireland.

Infections

Pseudomonas

41.4% of people aged 16 and over have chronic Pseudomonas. The median age of people with chronic Pseudomonas was 24 years in 2008, compared to 29 in 2018.

88.9% of people with chronic Pseudomonas were on inhaled antibiotic therapy in 2018, compared to 76.1% in 2008.

Non-tuberculous mycobacterium (NTM)

NTM has increased from 6% to 7% in the past year. 49% of people recorded as having NTM were being treated, which is 10% ​less than in 2017.

Aspergillus

Aspergillus is reported in 8.9% of people.

Allergic bronchopulmonary aspergillosis (ABPA), an immune response to Aspergillus infection, has reduced in prevalence from 10.5% since 2013 to 7.2%.

Median predicted survival age

For people born today, using 2014-2018 data, median predicted survival age is ​47.3 years old. ​The median predicted survival age for females ​(44.1) ​is 7 years lower than males ​(51.0)​.

Of the 137 people with CF who died in 2018, the median age of death was 32 years old.

Pregnancy

45 men with cystic fibrosis became fathers.

65 women with cystic fibrosis became mothers.

Mucus thinners

32.9% of people were on hypertonic saline in 2018, compared to 5% in 2008.

65% of people were on DNase in 2018, compared to 37% in 2008.

IV antibiotics

44.7% of people had at least one course of IV antibiotics in 2018. 37.7% of these were administered in hospital, and 24% ​were administered at home.

Cystic fibrosis-related diabetes (CFRD)

11.4% of children aged 10-15 were on CFRD treatment.

33.8% of adults 16 and over were on CFRD treatment.

Burden of treatment

79.4% of people with CF were on at least one form of inhaled therapy, and 20.6% of people were on no inhaled therapy.

People on CFTR modifiers

612 people were on ivacaftor (Kalydeco)

372 people were on lumacaftor/ivacaftor (Orkambi)

30 people were on tezacaftor/ivacaftor (Symkevi)

Transplant

2008

126 people were being evaluated for a transplant, 55 people were accepted and 16 had a double lung transplant.

2018

247 people were being evaluated for a transplant, 104 people were accepted and 58 had a double lung transplant.

Research we're funding

The Trust is committed to funding and supporting cutting-edge research to find new and better treatments for cystic fibrosis. Take a look at some of that work and the progress that is being made.